Precis Future Med.  2021 Sep;5(3):142-148. 10.23838/pfm.2021.00044.

Two cases of non-fluent variant primary progressive aphasia with different pathological diagnoses

Affiliations
  • 1Department of Neurology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
  • 2Samsung Alzheimer Research Center, Samsung Medical Center, Seoul, Korea
  • 3Neuroscience Center, Samsung Medical Center, Seoul, Korea
  • 4Department of Pathology and Translational Genomics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
  • 5Department of Neurology, Gyeongsang National University Changwon Hospital, Gyeongsang National University College of Medicine, Changwon, Korea
  • 6Department of Neurology, Kangwon National University Hospital, Kangwon National University School of Medicine, Chuncheon, Korea
  • 7Stem Cell & Regenerative Medicine Institute, Samsung Medical Center, Seoul, Korea
  • 8Department of Clinical Research Design and Evaluation, Samsung Advanced Institute for Health Sciences & Technology, Sungkyunkwan University, Seoul, Korea
  • 9Center for Clinical Epidemiology, Samsung Medical Center, Seoul, Korea
  • 10Department of Pathology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea

Abstract

Non-fluent variant primary progressive aphasia (nfvPPA), a subtype of frontotemporal lobar dementia syndrome, has been proven to have various pathological diagnoses. A 63-year-old woman and 71-year-old man separately visited our clinic for language dysfunction. Both patients showed non-fluent speech. The female patient showed personality change accompanied by language dysfunction, while the male patient had parkinsonian symptoms such as bradykinesia and cogwheel rigidity. Both patients were clinically diagnosed with nfvPPA. Several years after the first visit, the patients died, and a brain autopsy was performed. On postmortem examination, the female patient was pathologically diagnosed with Pick’s disease, while the male patient was diagnosed with progressive supranuclear palsy. Our report suggests that nfvPPA patients might show distinct clinical features depending on underlying pathologies.

Keyword

Autopsy; Pick disease of the brain; Primary progressive nonfluent aphasia; Supranuclear palsy, progressive
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